RESUMO
We report the case of a 14-month-old child with Kasabach-Merritt Syndrome, due to a giant liver hemangioma. The therapeutic approach consisted of peripheral transcatheter embolisation of the right hepatic artery with Ivalon microspheres without the addition of thrombogenic material. This procedure brought to a sensible permanent reduction of the size of the liver hemangioma with normalisation of the previous altered coagulation parameters after 6 years of follow-up.
Assuntos
Coagulação Intravascular Disseminada/etiologia , Embolização Terapêutica , Hemangioma/complicações , Hepatopatias/patologia , Arteriopatias Oclusivas , Coagulação Intravascular Disseminada/terapia , Feminino , Humanos , Lactente , Fígado/irrigação sanguínea , Fígado/patologia , Fígado/cirurgia , Hepatopatias/sangue , Hepatopatias/terapia , Síndrome , TomografiaAssuntos
Leucemia Prolinfocítica/tratamento farmacológico , Leucemia Prolinfocítica/terapia , Leucemia de Células T/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Terapia Combinada , Ciclofosfamida/administração & dosagem , Citarabina/administração & dosagem , Intervalo Livre de Doença , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Transplante de Células-Tronco Hematopoéticas , Humanos , Ifosfamida/administração & dosagem , Masculino , Metotrexato/administração & dosagem , Pessoa de Meia-Idade , Pentostatina/administração & dosagem , Prognóstico , Subpopulações de Linfócitos T/imunologia , Transplante Autólogo , Vincristina/administração & dosagemRESUMO
About 30% of patients with severe aplastic anaemia (SAA) unresponsive to one course of immunosuppressive (IS) therapy with antithymocyte or antilymphocyte globulin can achieve complete or partial remission after a second IS treatment. Among various second-line treatments, rabbit ATG (r-ATG) could represent a safe and effective alternative to horse ALG (h-ALG). In a multicentre study, 30 patients with SAA (17 males and 13 females, median age 21 years, range 2-67) not responding to a first course with h-ALG plus cyclosporin (CyA) and granulocyte colony stimulating factor (G-CSF), were given a second course using r-ATG (3.5 mg/kg/d for 5 d), CyA (5 mg/kg orally from day 1 to 180) and G-CSF (5 microg/kg subcutaneously from day 1 to 90). The median interval between first and second treatment was 151 d (range 58-361 d). No relevant side-effects were observed, but one patient died early during treatment because of sepsis. Overall response, defined as transfusion independence, was achieved in 23/30 (77%) patients after a median time of 95 d (range 14-377). Nine patients (30%) achieved complete remission (neutrophils >/=2.0 x 109/l, haemoglobin >/=11 g/dl and platelets >/=100 x 109/l). The overall survival rate was 93% with a median follow-up of 914 d (range 121-2278). So far, no patient has relapsed. Female gender was significantly associated with a poorer likelihood to respond (P = 0.0006). These data suggest that r-ATG is a safe and effective alternative to h-ALG for SAA patients unresponsive to first-line IS treatment.